What is dural ectasia?
by Ellen England (eeengland)

Here's my understanding.  The dura is a membrane inside your skull and spinal column that holds the cerebral spinal fluid which cushions your brain and spinal cord.  The dura is made up of connective tissue and consequently in Marfans it can get stretched out.  It can form a little pouch or dilitation which is known as dural ectasia.  As time goes by and this pouch continues to grow it can actually wear the bone away, from within the spine, and can sometimes protrude into the abdomen.

My neurologist told me that the normal diameter of the dura down in the area near my tailbone where my dural ectasia is, is about 1 - 2 cm.  Mine is 5-7 cm wide and 3-4 cm front to back.  He said mine hasn't come through the bone though.  I am prone to lower back pain and also often get achy legs which I attribute to the dural ectasia.  The worst symptom for me though happens under conditions of severe stress--I'll suddenly get a monster headache which is caused by drainage of the cerebral spinal fluid--the same sort of headache you get if you have a spinal tap.  The only way to make it go away is to lie down flat for awhile--30 minutes is usually enough for the worst of it to go away. These headaches aren't anything like migraines (which I also get sometimes). I don't know why stress causes the CSF leakage, but I have talked to a number of other Marfans who also report similar headaches.

I think it's most common for dural ectasias to appear in the lower spine, but I know of one person whose dural ectasia is higher up on her spine--I think maybe around her kidney area.

For those who are interested in more information, you might want to look up this article:
Pyeritz RE; Fishman EK; Bernhardt BA; Siegelman SS.
Dural ectasia is a common feature of the Marfan syndrome.
American Journal of Human Genetics, 1988 Nov, 43(5):726-32.

(Note from Jeanette: Many U.S. public libraries can obtain copies of articles from journals that your library doesn't own through an Inter-Library Loan process. Check with the reference desk at your local public library).

Dural ectasia in an eleven-year-old boy
by Carol Rudes (crudes)

Dural Ectasia is an "outpouching" of the dura that surrounds the spinal cord. The dura is made of guess what...connective tissue. In a "normal person" the dura and the enclosed spinal cord is a straight up and down tube.The dura holds the spinal fluid surrounding the spinal cord. In MRI studies of many Marfan persons the dura shows an outpouching around the bottom of the dura in the sacral area. Most often it does not cause undue pain or it may cause lower back and leg pain. However when it erodes the vertebrae and (gals remember studies have shown that Marfan women are more prone to osteoporosis as well) it can cause severe pain in odd areas including abdomen from the nerves radiating out.

Some people have experience of cerebral spinal fluid leaks which can cause headaches although they are usually cured by lying flat for 30 min or so. To date there is no "for sure surgical fix"for dural ectasia as the connective tissue is so delicate that the general  "Marfan" expert consensus is that it may cause more harm than good and at best be a tempory fix. However some poeple have had other surgical experiences who have written into this list for ectasias that had to be drained. That is something a little different than surgery to cordon off the outpouchings of the dural sacs. It would be interesting to gather as much anecdotal info on dural ectasias as possible -so if anyone has anything else to offer please do so.

I will tell that the horendous incredible pain that my son experienced from his multiple dural ectasias was brought under control to a major extent through the use of a drug called Tegretol. Tegretol is used to treat another condition called trigeminol neuralgia or sometimes called "Tic Doloureaux". It is a sudden excruciating nerve pain of the face. It is one of the oldest known reported medical pain conditions. Tegretol is ite effective for this and thus the jump to try it on my son for his convulsive nerve pain from the multiple Dural Ectasias. But remember this was a severe unusual case.

The other odd presentation about Matt's dural ectasias is that all the symptoms first appeared exactly as those of an abdomina aortic dissection. Severe back pain, severe abdominal pain and head ache. When asked the question "Is this like any other pain you have ever experienced?" the answer was NO! I have to tell that large amounts of morphine, demarol and even tried Toradol in the ER all day, but nothing brought his pain under control. Of course the first thing the cardiologist did was an echo, then a CT scan. She noticed the dural ectasias and felt that it was too significant a finding not to have something to do with his severe pain. This was after we eliminated any finding of abdominal aortic dissection) Matt was hospitalized with a morphine pump actually used another morphine like drug called Delauded, and still the pain was not controlled. It took several days to get the time in the scanner to have a full spinal and brain mri done. That is when the multiple ectasias showed up from cervical to sacral area down his spine. There was also ectasias around his optic nerves on the eyes, but that does not cause pain as there are no nerves in that area.

After several days of intense abdominal, back and head pain the incredible convulsive like spasms started. They were a like someone was putting a stun gun to his spine. They sometimes occurred up to 17 times in 1 day. The investigative story goes on, but as I said before the ultimate conclusion was that Tegretol (currently 400mg per day) brought that part of the pain under control. We hope to reduce the dose slightly within a couple of weeks.

This was an extremely debilitating experience, worse than any of his heart or orthopeadic surgeries to date.

Matt does have Infantile Marfan Syndrome, so probably that is why his case was a bit more extreme than one normally hears about.